{"id":"https://openalex.org/W2993535209","doi":null,"title":"Sickle cell disease: current treatment and emerging therapies.","display_name":"Sickle cell disease: current treatment and emerging therapies.","publication_year":2019,"publication_date":"2019-11-01","ids":{"openalex":"https://openalex.org/W2993535209","mag":"2993535209","pmid":"https://pubmed.ncbi.nlm.nih.gov/31809007"},"language":"en","primary_location":{"id":"pmid:31809007","doi":null,"is_oa":false,"landing_page_url":"https://pubmed.ncbi.nlm.nih.gov/31809007","pdf_url":null,"source":{"id":"https://openalex.org/S4306525036","display_name":"PubMed","issn_l":null,"issn":null,"is_oa":false,"is_in_doaj":false,"is_core":false,"listed_in":[],"host_organization":"https://openalex.org/I1299303238","host_organization_name":"National Institutes of Health","host_organization_lineage":["https://openalex.org/I1299303238"],"host_organization_lineage_names":[],"type":"repository"},"license":null,"license_id":null,"version":"publishedVersion","is_accepted":true,"is_published":true,"raw_source_name":"The American journal of managed care","raw_type":"Journal Article"},"volume":null,"type":"article","indexed_in":["pubmed"],"open_access":{"is_oa":false,"oa_status":"closed","oa_url":null,"any_repository_has_fulltext":false},"authorships":[{"author_position":"first","author":{"id":"https://openalex.org/A5043948649","display_name":"Lynne Neumayr","orcid":"https://orcid.org/0000-0003-1579-5865","observed_orcids":["https://orcid.org/0000-0003-1579-5865"]},"institutions":[],"countries":[],"is_corresponding":false,"raw_author_name":"Lynne D Neumayr","raw_affiliation_strings":[],"raw_orcid":null,"affiliations":[]},{"author_position":"middle","author":{"id":"https://openalex.org/A5001812366","display_name":"Carolyn C. Hoppe","orcid":"https://orcid.org/0000-0001-9213-0547","observed_orcids":["https://orcid.org/0000-0001-9213-0547"]},"institutions":[],"countries":[],"is_corresponding":false,"raw_author_name":"Carolyn C Hoppe","raw_affiliation_strings":[],"raw_orcid":null,"affiliations":[]},{"author_position":"last","author":{"id":"https://openalex.org/A5051820244","display_name":"Clark Brown","orcid":"https://orcid.org/0000-0003-0674-0213","observed_orcids":["https://orcid.org/0000-0003-0674-0213"]},"institutions":[{"id":"https://openalex.org/I4210139024","display_name":"Scottish Rite Hospital","ror":"https://ror.org/04cb32210","country_code":"US","type":"healthcare","lineage":["https://openalex.org/I4210139024","https://openalex.org/I4210146983"]}],"countries":["US"],"is_corresponding":false,"raw_author_name":"Clark Brown","raw_affiliation_strings":["Aflac Cancer and Blood Disorders Center, Scottish Rite Hospital, 5461 Meridian Mark Rd NE, Medical Office Building Suite 400, Atlanta, GA 30342. Email: clark.brown@choa.org","Aflac Cancer and Blood Disorders Center, Scottish Rite Hospital, 5461 Meridian Mark Rd NE, Medical Office Building Suite 400, Atlanta, GA 30342. Email:"],"raw_orcid":null,"affiliations":[{"raw_affiliation_string":"Aflac Cancer and Blood Disorders Center, Scottish Rite Hospital, 5461 Meridian Mark Rd NE, Medical Office Building Suite 400, Atlanta, GA 30342. Email: clark.brown@choa.org","institution_ids":["https://openalex.org/I4210139024"]},{"raw_affiliation_string":"Aflac Cancer and Blood Disorders Center, Scottish Rite Hospital, 5461 Meridian Mark Rd NE, Medical Office Building Suite 400, Atlanta, GA 30342. Email:","institution_ids":["https://openalex.org/I4210139024"]}]}],"institutions":[],"countries_distinct_count":1,"institutions_distinct_count":1,"corresponding_author_ids":[],"corresponding_institution_ids":[],"apc_list":null,"apc_paid":null,"fwci":1.9224,"has_fulltext":false,"cited_by_count":42,"citation_normalized_percentile":{"value":0.85370052,"is_in_top_1_percent":false,"is_in_top_10_percent":false},"cited_by_percentile_year":{"min":95,"max":99},"biblio":{"volume":"25","issue":"18 Suppl","first_page":"S335","last_page":"S343"},"is_retracted":false,"is_paratext":false,"is_xpac":false,"primary_topic":{"id":"https://openalex.org/T10554","display_name":"Hemoglobinopathies and Related Disorders","score":1.0,"subfield":{"id":"https://openalex.org/subfields/2716","display_name":"Genetics"},"field":{"id":"https://openalex.org/fields/27","display_name":"Medicine"},"domain":{"id":"https://openalex.org/domains/4","display_name":"Health Sciences"}},"topics":[{"id":"https://openalex.org/T10554","display_name":"Hemoglobinopathies and Related Disorders","score":1.0,"subfield":{"id":"https://openalex.org/subfields/2716","display_name":"Genetics"},"field":{"id":"https://openalex.org/fields/27","display_name":"Medicine"},"domain":{"id":"https://openalex.org/domains/4","display_name":"Health Sciences"}},{"id":"https://openalex.org/T12094","display_name":"Hemoglobin structure and function","score":0.0,"subfield":{"id":"https://openalex.org/subfields/1307","display_name":"Cell Biology"},"field":{"id":"https://openalex.org/fields/13","display_name":"Biochemistry, Genetics and Molecular Biology"},"domain":{"id":"https://openalex.org/domains/1","display_name":"Life Sciences"}},{"id":"https://openalex.org/T11660","display_name":"Blood groups and transfusion","score":0.0,"subfield":{"id":"https://openalex.org/subfields/2720","display_name":"Hematology"},"field":{"id":"https://openalex.org/fields/27","display_name":"Medicine"},"domain":{"id":"https://openalex.org/domains/4","display_name":"Health Sciences"}}],"keywords":[{"id":"https://openalex.org/keywords/sickle-cell-disease","display_name":"sickle cell disease","score":1.0},{"id":"https://openalex.org/keywords/hydroxyurea","display_name":"hydroxyurea","score":0.9070000052452087},{"id":"https://openalex.org/keywords/glutamine","display_name":"glutamine","score":0.9779999852180481},{"id":"https://openalex.org/keywords/vessel-occlusion","display_name":"vascular occlusion","score":0.8270000219345093},{"id":"https://openalex.org/keywords/hemoglobin-polymerization","display_name":"hemoglobin polymerization","score":0.8690000176429749},{"id":"https://openalex.org/keywords/oxidative-stress","display_name":"oxidative stress","score":0.6919999718666077},{"id":"https://openalex.org/keywords/endothelial-dysfunction","display_name":"endothelial dysfunction","score":0.8730000257492065},{"id":"https://openalex.org/keywords/chronic-hemolytic-anemia","display_name":"chronic hemolytic anemia","score":0.7490000128746033},{"id":"https://openalex.org/keywords/acute-chest-syndrome","display_name":"acute chest syndrome","score":0.8529999852180481},{"id":"https://openalex.org/keywords/stroke","display_name":"stroke","score":0.7889999747276306},{"id":"https://openalex.org/keywords/pulmonary-hypertension","display_name":"pulmonary hypertension","score":0.7250000238418579},{"id":"https://openalex.org/keywords/curative-treatment","display_name":"curative treatment","score":0.5509999990463257},{"id":"https://openalex.org/keywords/novel-therapies","display_name":"new drugs","score":0.5519999861717224}],"concepts":[{"id":"https://openalex.org/C71924100","wikidata":"https://www.wikidata.org/wiki/Q11190","display_name":"Medicine","level":0,"score":0.9494374990463257},{"id":"https://openalex.org/C2780976302","wikidata":"https://www.wikidata.org/wiki/Q4677915","display_name":"Acute chest syndrome","level":4,"score":0.8336139917373657},{"id":"https://openalex.org/C2779134260","wikidata":"https://www.wikidata.org/wiki/Q12136","display_name":"Disease","level":2,"score":0.549460232257843},{"id":"https://openalex.org/C2780645631","wikidata":"https://www.wikidata.org/wiki/Q671554","display_name":"Stroke (engine)","level":2,"score":0.5395104289054871},{"id":"https://openalex.org/C2778248108","wikidata":"https://www.wikidata.org/wiki/Q5445","display_name":"Anemia","level":2,"score":0.5065666437149048},{"id":"https://openalex.org/C10162356","wikidata":"https://www.wikidata.org/wiki/Q1135939","display_name":"Pathophysiology","level":2,"score":0.5001046657562256},{"id":"https://openalex.org/C2780972559","wikidata":"https://www.wikidata.org/wiki/Q550061","display_name":"Endothelial dysfunction","level":2,"score":0.43084716796875},{"id":"https://openalex.org/C126322002","wikidata":"https://www.wikidata.org/wiki/Q11180","display_name":"Internal medicine","level":1,"score":0.40791094303131104},{"id":"https://openalex.org/C177713679","wikidata":"https://www.wikidata.org/wiki/Q679690","display_name":"Intensive care medicine","level":1,"score":0.38322556018829346},{"id":"https://openalex.org/C2778620579","wikidata":"https://www.wikidata.org/wiki/Q185034","display_name":"Sickle cell anemia","level":3,"score":0.34745949506759644},{"id":"https://openalex.org/C60644358","wikidata":"https://www.wikidata.org/wiki/Q128570","display_name":"Bioinformatics","level":1,"score":0.32446908950805664},{"id":"https://openalex.org/C78519656","wikidata":"https://www.wikidata.org/wiki/Q101333","display_name":"Mechanical engineering","level":1,"score":0.0},{"id":"https://openalex.org/C127413603","wikidata":"https://www.wikidata.org/wiki/Q11023","display_name":"Engineering","level":0,"score":0.0},{"id":"https://openalex.org/C86803240","wikidata":"https://www.wikidata.org/wiki/Q420","display_name":"Biology","level":0,"score":0.0}],"mesh":[{"descriptor_ui":"D000755","descriptor_name":"Anemia, Sickle Cell","qualifier_ui":"Q000453","qualifier_name":"epidemiology","is_major_topic":false},{"descriptor_ui":"D000755","descriptor_name":"Anemia, Sickle Cell","qualifier_ui":"Q000503","qualifier_name":"physiopathology","is_major_topic":true},{"descriptor_ui":"D000755","descriptor_name":"Anemia, Sickle Cell","qualifier_ui":"Q000628","qualifier_name":"therapy","is_major_topic":true},{"descriptor_ui":"D000986","descriptor_name":"Antisickling Agents","qualifier_ui":"Q000627","qualifier_name":"therapeutic use","is_major_topic":true},{"descriptor_ui":"D005260","descriptor_name":"Female","qualifier_ui":null,"qualifier_name":null,"is_major_topic":false},{"descriptor_ui":"D006801","descriptor_name":"Humans","qualifier_ui":null,"qualifier_name":null,"is_major_topic":false},{"descriptor_ui":"D006918","descriptor_name":"Hydroxyurea","qualifier_ui":"Q000627","qualifier_name":"therapeutic use","is_major_topic":true},{"descriptor_ui":"D008297","descriptor_name":"Male","qualifier_ui":null,"qualifier_name":null,"is_major_topic":false},{"descriptor_ui":"D014481","descriptor_name":"United States","qualifier_ui":"Q000453","qualifier_name":"epidemiology","is_major_topic":false},{"descriptor_ui":"D017707","descriptor_name":"Erythrocyte Transfusion","qualifier_ui":"Q000379","qualifier_name":"methods","is_major_topic":true},{"descriptor_ui":"D035703","descriptor_name":"Therapies, Investigational","qualifier_ui":"Q000379","qualifier_name":"methods","is_major_topic":true}],"locations_count":2,"locations":[{"id":"pmid:31809007","doi":null,"is_oa":false,"landing_page_url":"https://pubmed.ncbi.nlm.nih.gov/31809007","pdf_url":null,"source":{"id":"https://openalex.org/S4306525036","display_name":"PubMed","issn_l":null,"issn":null,"is_oa":false,"is_in_doaj":false,"is_core":false,"listed_in":[],"host_organization":"https://openalex.org/I1299303238","host_organization_name":"National Institutes of Health","host_organization_lineage":["https://openalex.org/I1299303238"],"host_organization_lineage_names":[],"type":"repository"},"license":null,"license_id":null,"version":"publishedVersion","is_accepted":true,"is_published":true,"raw_source_name":"The American journal of managed care","raw_type":"Journal Article"},{"id":"mag:2993535209","doi":null,"is_oa":false,"landing_page_url":"https://pubmed.ncbi.nlm.nih.gov/31809007","pdf_url":null,"source":{"id":"https://openalex.org/S4306525036","display_name":"PubMed","issn_l":null,"issn":null,"is_oa":false,"is_in_doaj":false,"is_core":false,"listed_in":[],"host_organization":"https://openalex.org/I1299303238","host_organization_name":"National Institutes of Health","host_organization_lineage":["https://openalex.org/I1299303238"],"host_organization_lineage_names":[],"type":"repository"},"license":null,"license_id":null,"version":null,"is_accepted":false,"is_published":false,"raw_source_name":"PubMed","raw_type":null}],"best_oa_location":null,"sustainable_development_goals":[{"display_name":"Good health and well-being","id":"https://metadata.un.org/sdg/3","score":1.0}],"study_designs":[],"awards":[],"funders":[],"has_content":{"pdf":false,"grobid_xml":false},"content_urls":null,"referenced_works_count":0,"referenced_works":[],"related_works":["https://openalex.org/W4214873705","https://openalex.org/W4283837991","https://openalex.org/W2064466508","https://openalex.org/W2899915044","https://openalex.org/W3126283762","https://openalex.org/W2555622781","https://openalex.org/W2325332921","https://openalex.org/W4289222968","https://openalex.org/W2134602529","https://openalex.org/W4408038311"],"abstract_inverted_index":{"Sickle":[0],"cell":[1],"disease":[2,220],"(SCD)":[3],"is":[4,24,49,66],"among":[5],"the":[6,12,20,155,173,176,182],"most":[7],"common":[8],"genetic":[9],"diseases":[10],"in":[11,36,165,192],"United":[13,21],"States,":[14,22],"affecting":[15,60],"approximately":[16],"100,000":[17],"people.":[18],"In":[19],"SCD":[23,48,160,201],"characterized":[25,50],"by":[26,51],"a":[27,76,146,189,217],"shortened":[28],"life":[29],"expectancy":[30],"of":[31,71,78,96,108,149,175,179,200],"only":[32,156],"about":[33],"50":[34],"years":[35,185],"severe":[37],"subtypes,":[38],"significant":[39],"quality-of-life":[40],"impairments,":[41],"and":[42,46,56,87,98,106,117,137,145,203,214],"increased":[43,103],"healthcare":[44],"utilization":[45],"spending.":[47],"chronic":[52,118,129],"hemolytic":[53],"anemia,":[54,135],"vaso-occlusion,":[55,84],"progressive":[57],"vascular":[58],"injury":[59,89],"multiple":[61,122],"organ":[62,123],"systems.":[63],"The":[64],"pathophysiology":[65,202],"directly":[67],"related":[68],"to":[69,75,212],"polymerization":[70],"deoxygenated":[72],"hemoglobin,":[73],"leading":[74],"cascade":[77],"pathologic":[79],"events":[80],"including":[81,125],"erythrocyte":[82],"sickling,":[83],"tissue":[85],"ischemia,":[86],"reperfusion":[88],"as":[90,92],"well":[91],"hemolysis,":[93],"abnormal":[94],"activation":[95,107],"inflammatory":[97],"oxidative":[99,104],"pathways,":[100],"endothelial":[101],"dysfunction,":[102,142],"stress,":[105],"coagulation":[109],"pathways.":[110],"These":[111],"multifactorial":[112],"abnormalities":[113],"have":[114],"both":[115],"acute":[116,126,132,177],"clinical":[119,151],"consequences":[120],"across":[121],"systems,":[124],"pain":[127,130],"episodes,":[128],"syndromes,":[131],"chest":[133],"syndrome,":[134],"stroke":[136],"silent":[138],"cerebral":[139],"infarcts,":[140],"cognitive":[141],"pulmonary":[143],"hypertension,":[144],"wide":[147],"range":[148],"other":[150],"consequences.":[152],"Hydroxyurea":[153],"was":[154,170],"approved":[157,171],"treatment":[158],"for":[159,161,172,216],"nearly":[162],"2":[163],"decades;":[164],"2017,":[166],"L-glutamine":[167],"oral":[168],"powder":[169],"prevention":[174],"complications":[178],"SCD.":[180],"During":[181],"last":[183],"several":[184],"there":[186],"has":[187,222],"been":[188,224],"dramatic":[190],"increase":[191],"research":[193],"into":[194],"treatments":[195],"that":[196,208,221],"address":[197],"distinct":[198],"elements":[199],"even":[204],"new":[205,210],"curative":[206],"approaches":[207],"provide":[209],"hope":[211],"patients":[213],"physicians":[215],"clinically":[218],"consequential":[219],"long":[223],"neglected.":[225]},"counts_by_year":[{"year":2025,"cited_by_count":4},{"year":2024,"cited_by_count":10},{"year":2023,"cited_by_count":13},{"year":2022,"cited_by_count":3},{"year":2021,"cited_by_count":8},{"year":2020,"cited_by_count":3}],"updated_date":"2026-10-31T12:09:11.096373","created_date":"2019-12-13T00:00:00"}